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Case reports: Central nervous system involvement in patients with newly diagnosed multiple myeloma
Jinghua Liu1,2, Jing Shen3, Daihong Liu1
1Department of Hematology, The Fifth Medical Center of Chinese PLA General Hospital, Beijing, China.
Abstract:
Multiple myeloma with central nervous system involvement (CNS-MM) is rare, having a poor outcome and occurring in newly diagnosed or relapsed/refractory patients. The current report concerns 3 cases of newly diagnosed MM patients who presented with skull-derived plasmacytomas. Case 1 was a 54-year-old female patient with immunoglobulin D (IgD) subtype who developed extramedullary lesions from the sphenoid and occipital bones and the sphenoid sinus. Cases 2 and 3 had IgA subtype with left or bilateral frontal area lesions. Case 1 was treated with bortezomib, cyclophosphamide and dexamethasone (VCD) as the initial chemotherapy regimen and with bortezomib, lenalidomide, pegylated liposomal doxorubicin and dexamethasone (DVD-R) as the second line regimen. Whole-brain irradiation and intrathecal injection were given but the patient died within 9 months due to disease progression. Case 2 was treated with bortezomib, lenalidomide and dexamethasone (VRD) and received autologous hematopoietic stem cell transplantation (auto-HSCT) with a conditioning regimen of cyclophosphamide, etoposide and melphalan (CEM). Case 3 received DVD-R initially and auto-HSCT with a conditioning regimen of busulfan, cyclophosphamide, and etoposide (BuCyE). Cases 2 and 3 survived until the last follow-up more than 3 years later. Auto-HSCT with modified conditioning regimen as consolidation therapy improved the prognosis of CNS-MM.
Insights
Central nervous system involvement in multiple myeloma (CNS-MM) is rare. Autologous hematopoietic stem cell transplantation (auto-HSCT) with modified conditioning regimens improved prognosis in CNS-MM patients with skull plasmacytomas.
Area of Science:
- Hematology
- Oncology
- Neuro-oncology
Background:
- Multiple myeloma with central nervous system involvement (CNS-MM) is a rare complication with a poor prognosis.
- CNS-MM can occur in newly diagnosed or relapsed/refractory patients.
- Skull-derived plasmacytomas are an uncommon presentation of CNS-MM.
Observation:
- This report details three newly diagnosed multiple myeloma patients presenting with skull plasmacytomas.
- Case 1 (IgD subtype) experienced extensive extramedullary lesions and succumbed to disease progression despite aggressive treatment including whole-brain irradiation and intrathecal chemotherapy.
- Cases 2 and 3 (IgA subtype) with frontal bone lesions showed prolonged survival exceeding three years following autologous hematopoietic stem cell transplantation (auto-HSCT) with modified conditioning regimens.
Findings:
- Treatment strategies varied, with Case 1 receiving standard chemotherapy and radiotherapy, while Cases 2 and 3 underwent auto-HSCT.
- The use of modified conditioning regimens for auto-HSCT in Cases 2 and 3 was associated with significantly improved outcomes.
- Autologous hematopoietic stem cell transplantation (auto-HSCT) appears to be a crucial consolidation therapy for CNS-MM with skull involvement.
Implications:
- Modified autologous hematopoietic stem cell transplantation (auto-HSCT) regimens offer a promising therapeutic strategy for improving survival in CNS-MM patients with skull plasmacytomas.
- Further research is warranted to optimize conditioning regimens for auto-HSCT in CNS-MM.
- Early identification and aggressive management, including potentially auto-HSCT, are critical for improving the prognosis of this rare but aggressive complication of multiple myeloma.
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